OP0236 RISK FACTORS FOR SSc-ILD PROGRESSION USING THE NEW PPF GUIDELINE AND INBUILD PF-ILD CRITERIA
Authors: A. M. Hoffmann-Vold, L. Petelytska, H. Fretheim, I. Barua, M. O. Becker, H. J. Bjørkekjær, C. Brunborg, C. Bruni, P. P. Diep, R. Dobrota, M. Durheim, M. Elhai, S. Jordan, E. Langballe, Ø. Midtvedt, C. Mihai, Ø. Molberg, O. Distler
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Interstitial lung disease (ILD) progression can be assessed using different definitions. Declining forced vital capacity (FVC) is the simplest and most accepted. Both progressive pulmonary fibrosis (PPF) as defined by the 2022 ATS/ERS/JRS/ALAT guideline, and progressive fibrosing ILD (PF-ILD) as used in INBUILD, define progressive disease by a composite worsening of lung function, respiratory symptoms and changes on high resolution chest tomography (HRCT), but with differences in the specific criteria. These new definitions have not been applied and compared in SSc-ILD yet. <h3>Objectives</h3> To identify variables predicting SSc-ILD progression using three different definitions of progressive disease. <h3>Methods</h3> We included all SSc patients from the Oslo and Zurich cohorts who had ILD on HRCT. ILD progression was defined as (A) PPF guideline criteria with worsening in 2/3 domains over 12 months including (1) respiratory symptoms; (2) absolute decline in FVC &gt;5% or in DLCO &gt;10% and (3) radiological disease progression. (B) PF-ILD INBUILD criteria with (1) FVC decline ≥10%, (2) FVC decline 5-9% and worsening of respiratory symptoms or increased lung fibrosis on HRCT, or (3) worsening of respiratory symptoms and increased lung fibrosis within 24 months. We compared these to ILD progression defined as FVC decline &gt;5%. Variables for ILD progression chosen by expert opinion were tested in multivariable logistic models adjusted for age, sex and treatment and each model was tested using area under curve (AUC) with an AUC of 0.7 defined as acceptable. <h3>Results</h3> Of 231 included SSc-ILD patients from Oslo and Zurich 43 (19%) fulfilled the PPF, 89 (39%) the PF-ILD INBUILD definitions and 71 (31%) showed FVC decline &gt;5%. The final multivariable logistic model predictive for ILD progression defined as FVC&gt;5% decline included baseline FVC, respiratory symptoms (defined as functional class 3 and 4) and the presence of anti-topoisomerase I antibody (ATA). The model was considered as acceptable (AUC=0.70). The model for PF-ILD included diffuse cutaneous SSc (dcSSc), baseline FVC, respiratory symptoms and six-minute walking distance and had an acceptable AUC of 0.73. PPF was only predicted by reduced six minute walking distance, and the model was not acceptable (AUC=0.64). <h3>Conclusion</h3> Progression is common in SSc-ILD, regardless of definition, but the PPF guideline criteria identified fewer progressors than FVC decline &gt;5% and PF-ILD. We identified different variables predicting progression of FVC decline &gt;5% and PF-ILD but could not identify a model with acceptable performance for PPF. This raises concerns about which definition to use in clinical practice and future studies. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> Anna-Maria Hoffmann-Vold Speakers bureau: Boehringer Ingelheim, Jannsen, Medscape, Merck Sharp &amp; Dohme and Roche, Consultant of: ARXX, Boehringer Ingelheim, Genentech, Jannsen, Medscape, Merck Sharp &amp; Dohme and Roche, Grant/research support from: Boehringer Ingelheim, Jannsen, Liubov Petelytska: None declared, Håvard Fretheim Speakers bureau: Boehringer Ingelheim, Grant/research support from: Jannsen, Imon Barua: None declared, Mike O. Becker Speakers bureau: Mepha, Bayer, MSD, GSK, Amgen, Novartis and Vifor outside the submitted work., Hilde Jenssen Bjørkekjær Grant/research support from: Jannsen, Cathrine Brunborg: None declared, Cosimo Bruni Speakers bureau: Eli-Lilly, Consultant of: Boehringer Ingelheim, Grant/research, Grant/research support from: Gruppo Italiano Lotta alla Sclerodermia (GILS), European Scleroderma Trials and Research Group (EUSTAR), Foundation for research in Rheumatology (FOREUM), Scleroderma Clinical Trials Consortium (SCTC). Educational grants from AbbVie, Phuong Phuong Diep Speakers bureau: Boehringer-Ingelheim, Consultant of: Boehringer-Ingelheim, Rucsandra Dobrota Speakers bureau: Actelion and Boehringer-Ingelheim, Grant/research support from: Articulum Fellowship, sponsored by Pfizer (2013-2014), Actelion, Amgen, outside the submitted work., Michael Durheim Speakers bureau: Boehringer Ingelheim, Grant/research support from: Boehringer Ingelheim, Muriel Elhai Speakers bureau: BMS, Grant/research support from: Support for travel (Janssen), Suzana Jordan: None declared, Emily Langballe: None declared, Øyvind Midtvedt: None declared, Carina Mihai Speakers bureau: Boehringer Ingelheim, Consultant of: Boehringer Ingelheim, Janssen, MED Talks Switzerland, Mepha, and PlayToKnow AG, Grant/research support from: Boehringer Ingelheim, Øyvind Molberg: None declared, Oliver Distler Speakers bureau: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant, Sanofi and Topadur. Patent issued "mir-29 for the treatment of systemic sclerosis" (US8247389, EP2331143)., Consultant of: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, iQvia, Horizon, Inventiva, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant, Sanofi and Topadur., Grant/research support from: BI, Kymera, Mitsubishi Tanabe.